Tweaking progranulin expression: therapeutic avenues and opportunities

J Terryn, CM Verfaillie, P Van Damme - Frontiers in Molecular …, 2021 - frontiersin.org
Frontotemporal dementia (FTD) is a neurodegenerative disease, leading to behavioral
changes and language difficulties. Heterozygous loss-of-function mutations in progranulin …

Progranulin mutations in ubiquitin-positive frontotemporal dementia linked to chromosome 17q21

M Cruts, S Kumar-Singh… - Current Alzheimer …, 2006 - ingentaconnect.com
Two genetically distinct types of frontotemporal dementia (FTD) are linked to chromosome
17q21. FTD with parkinsonism (FTDP-17) results from mutations in the gene encoding …

Reduction of microglial progranulin does not exacerbate pathology or behavioral deficits in neuronal progranulin-insufficient mice

AE Arrant, AJ Filiano, AR Patel, MQ Hoffmann… - Neurobiology of …, 2019 - Elsevier
Loss-of-function mutations in progranulin (GRN), most of which cause progranulin
haploinsufficiency, are a major autosomal dominant cause of frontotemporal dementia …

Molecular characterization of novel progranulin (GRN) mutations in frontotemporal dementia

O Mukherjee, J Wang, M Gitcho, S Chakraverty… - Human …, 2008 - Wiley Online Library
Frontotemporal dementia (FTD) is a clinical term encompassing dementia characterized by
the presence of two major phenotypes: 1) behavioral and personality disorder, and 2) …

Brain progranulin expression in GRN-associated frontotemporal lobar degeneration

AS Chen-Plotkin, J Xiao, F Geser, M Martinez-Lage… - Acta …, 2010 - Springer
Frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) is characterized by
progressive decline in behavior, executive function, and language. Progranulin (GRN) gene …

Progranulin protects against amyloid β deposition and toxicity in Alzheimer's disease mouse models

SS Minami, SW Min, G Krabbe, C Wang, Y Zhou… - Nature medicine, 2014 - nature.com
Haploinsufficiency of the progranulin (PGRN) gene (GRN) causes familial frontotemporal
lobar degeneration (FTLD) and modulates an innate immune response in humans and in …

Core neuropathological abnormalities in progranulin-deficient mice are penetrant on multiple genetic backgrounds

TL Petkau, A Hill, BR Leavitt - Neuroscience, 2016 - Elsevier
Loss-of-function mutations in the progranulin gene (GRN) are a common cause of familial
frontotemporal lobar degeneration (FTLD). A high degree of heterogeneity in the age-of …

Intracellular proteolysis of progranulin generates stable, lysosomal granulins that are haploinsufficient in patients with frontotemporal dementia caused by GRN …

CJ Holler, G Taylor, Q Deng, T Kukar - Eneuro, 2017 - eneuro.org
Homozygous or heterozygous mutations in the GRN gene, encoding progranulin (PGRN),
cause neuronal ceroid lipofuscinosis (NCL) or frontotemporal dementia (FTD), respectively …

MicroRNA-29b regulates the expression level of human progranulin, a secreted glycoprotein implicated in frontotemporal dementia

J Jiao, LD Herl, RV Farese Jr, FB Gao - PloS one, 2010 - journals.plos.org
Progranulin deficiency is thought to cause some forms of frontotemporal dementia (FTD), a
major early-onset age-dependent neurodegenerative disease. How progranulin (PGRN) …

Microglial NFκB-TNFα hyperactivation induces obsessive–compulsive behavior in mouse models of progranulin-deficient frontotemporal dementia

G Krabbe, SS Minami, JI Etchegaray… - Proceedings of the …, 2017 - National Acad Sciences
Frontotemporal dementia (FTD) is the second most common dementia before 65 years of
age. Haploinsufficiency in the progranulin (GRN) gene accounts for 10% of all cases of …